Supporting adults who have Prader-Willi syndrome : caregivers' perspectives on the ethical and practical dilemmas : a thesis presented in partial fulfillment of the requirements for the degree of Doctor of Philosophy in Psychology at Massey University, Wellington, New Zealand

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Massey University
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Prader-Willi Syndrome (PWS) is a complex genetic condition that typically results in learning impairment and hyperphagia, which is the drive to consume excessive quantities of food. Obesity often develops very rapidly and is life-threatening, presenting professional and familial support people with a distinctive set of ethical and practical dilemmas. These unique challenges can be characterised as a microcosm of a wider “safety versus autonomy” debate within human services. Little is known about the nature or efficacy of publically funded supports to adults who have PWS in Aotearoa/New Zealand. The aim of this thesis was to investigate the ethos of current services and some of the outcomes associated with them. In particular, it sought to illuminate the ways in which professional caregivers address the challenges they face in their work, according to their own reported perceptions of their role. The thesis begins with a review of the relevant international research literature, noting the dominance of clinical experience and anecdotes over research studies within it. This review has informed three investigations. First, a scoping study comprised of semi-structured interviews was conducted with professional caregivers in workplace-based focus groups. Second, an attempt was made to identify and survey all adults within Aotearoa/New Zealand who have PWS regarding the supports they received. Finally, a thematic analysis of the transcribed texts of interviews with individual caregivers was undertaken. These interviews concerned the ways in which caregivers of adults with PWS experienced, conceptualised and addressed the dilemmas inherent in their role. Findings indicated that there are grounds for serious concern about both the wellbeing of people with PWS in Aotearoa/New Zealand, and that of their caregivers. Significant levels of obesity were reported in almost all service users with PWS, despite the ecological controls over access to calories imposed on them by caregivers. It seems likely that some adults with PWS are at risk of serious harm while having their civil rights infringed to little or no effect. Caregiver participants characterised their role in terms of the challenges it presented them. The management of service users’ access to food was identified as only one of the sources of such challenge. Challenging behaviour among people with PWS was reported to generate the most significant and pressing difficulties. Caregivers reported responding to these challenges by imposing substitute decisions, especially in relation to food, despite awareness that such measures seemed to constitute a breach of ethical norms. They regarded compulsory ecological controls as necessary for the service users’ safety, but also as a compassionate response which prevented uncertainty in relation to food and hence spared service users with PWS from subsequent anxiety. Caregivers seemed in agreement with a view of client autonomy as relational and seemed to employ a dialogical process featuring stories and responses to stories in collective decision making. Despite the emphasis on individualism in contemporary services to people with disabilities, interviewees often seemed to prioritise corporate wellbeing, even when making decisions regarding access to food for a single individual with PWS. The conclusions which may be drawn from these studies are limited by a number of factors, notably small sample size. Nevertheless, they offer insights into the nature of the caregiving task and the concept of autonomy within human services. Findings form the basis of suggested innovations, both in caregiving practice and for future research.
Prader-Willi syndrome, Patients, Services for, New Zealand, Caregivers, Attitudes